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首页> 外文期刊>Cell metabolism >The human cytochrome c oxidase assembly factors SCO1 and SCO2 have regulatory roles in the maintenance of cellular copper homeostasis.
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The human cytochrome c oxidase assembly factors SCO1 and SCO2 have regulatory roles in the maintenance of cellular copper homeostasis.

机译：人类细胞色素c氧化酶装配因子SCO1和SCO2在维持细胞铜稳态中具有调节作用。

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摘要

Human SCO1 and SCO2 are metallochaperones that are essential for the assembly of the catalytic core of cytochrome c oxidase (COX). Here we show that they have additional, unexpected roles in cellular copper homeostasis. Mutations in either SCO result in a cellular copper deficiency that is both tissue and allele specific. This phenotype can be dissociated from the defects in COX assembly and is suppressed by overexpression of SCO2, but not SCO1. Overexpression of a SCO1 mutant in control cells in which wild-type SCO1 levels were reduced by shRNA recapitulates the copper-deficiency phenotype in SCO1 patient cells. The copper-deficiency phenotype reflects not a change in high-affinity copper uptake but rather a proportional increase in copper efflux. These results suggest a mitochondrial pathway for the regulation of cellular copper content that involves signaling through SCO1 and SCO2, perhaps by their thiol redox or metal-binding state.
机译：人SCO1和SCO2是金属伴侣蛋白，对于组装细胞色素C氧化酶（COX）的催化核心至关重要。在这里，我们证明了它们在细胞铜稳态中还具有其他意想不到的作用。任一SCO中的突变都会导致组织和等位基因特异性的细胞铜缺乏。此表型可以与COX装配中的缺陷分离，并可以通过SCO2（而非SCO1）的过表达来抑制。在野生型SCO1水平被shRNA降低的对照细胞中，SCO1突变体的过表达重现了SCO1患者细胞中的铜缺乏表型。缺铜表型不是反映高亲和力铜吸收的变化，而是铜流出的成比例增加。这些结果表明，用于调节细胞内铜含量的线粒体途径涉及通过SCO1和SCO2的信号传导，也许通过其硫醇氧化还原或金属结合状态来进行。

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